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Loss of glial fibrillary acidic protein marginally accelerates disease progression in a SOD1(H46R) transgenic mouse model of ALS.
Neurosci Res. 2011 Jul; 70(3):321-9.NR

Abstract

Glial fibrillary acidic protein (GFAP) is an intermediate filament protein that is highly expressed in reactive astrocytes. Increased production of GFAP is a hallmark of astrogliosis in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS). However, the physiological and pathological roles of GFAP, particularly in chronic neurodegenerative conditions, remain unclear. To address this issue, we here investigate whether absence of GFAP affects the phenotypic expression of motor neuron disease (MND) using an H46R mutant Cu/Zn superoxide dismutase-expressing mouse model of ALS (SOD1(H46R)). GFAP deficient SOD1(H46R) mice showed a significant shorter lifespan than SOD1(H46R) littermates. Further, at the end stage of disease, loss of GFAP resulted in increased levels of Vim and Aif1 mRNAs, encoding vimentin and allograft inflammatory factor 1 (AIF1), respectively, in the spinal cord, although no discernible differences in the levels and distribution of these proteins between SOD1(H46R) and GFAP-deficient SOD1(H46R) mice were observed. These results suggest that loss of GFAP in SOD1(H46R) mice marginally accelerates the disease progression by moderately enhancing glial cell activation. Our findings in a mouse model of ALS may have implication that GFAP is not necessary for the initiation of disease, but it rather plays some modulatory roles in the progression of ALS/MND.

Authors+Show Affiliations

Department of Neurology, Toho University Omori Medical Center, 6-11-1 Omori-nishi, Ota-ku, Tokyo 143-8541, Japan.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

21453731

Citation

Yoshii, Yasuhiro, et al. "Loss of Glial Fibrillary Acidic Protein Marginally Accelerates Disease Progression in a SOD1(H46R) Transgenic Mouse Model of ALS." Neuroscience Research, vol. 70, no. 3, 2011, pp. 321-9.
Yoshii Y, Otomo A, Pan L, et al. Loss of glial fibrillary acidic protein marginally accelerates disease progression in a SOD1(H46R) transgenic mouse model of ALS. Neurosci Res. 2011;70(3):321-9.
Yoshii, Y., Otomo, A., Pan, L., Ohtsuka, M., & Hadano, S. (2011). Loss of glial fibrillary acidic protein marginally accelerates disease progression in a SOD1(H46R) transgenic mouse model of ALS. Neuroscience Research, 70(3), 321-9. https://doi.org/10.1016/j.neures.2011.03.006
Yoshii Y, et al. Loss of Glial Fibrillary Acidic Protein Marginally Accelerates Disease Progression in a SOD1(H46R) Transgenic Mouse Model of ALS. Neurosci Res. 2011;70(3):321-9. PubMed PMID: 21453731.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Loss of glial fibrillary acidic protein marginally accelerates disease progression in a SOD1(H46R) transgenic mouse model of ALS. AU - Yoshii,Yasuhiro, AU - Otomo,Asako, AU - Pan,Lei, AU - Ohtsuka,Masato, AU - Hadano,Shinji, Y1 - 2011/03/29/ PY - 2011/01/11/received PY - 2011/02/21/revised PY - 2011/03/14/accepted PY - 2011/4/2/entrez PY - 2011/4/2/pubmed PY - 2012/5/5/medline SP - 321 EP - 9 JF - Neuroscience research JO - Neurosci Res VL - 70 IS - 3 N2 - Glial fibrillary acidic protein (GFAP) is an intermediate filament protein that is highly expressed in reactive astrocytes. Increased production of GFAP is a hallmark of astrogliosis in neurodegenerative diseases such as amyotrophic lateral sclerosis (ALS). However, the physiological and pathological roles of GFAP, particularly in chronic neurodegenerative conditions, remain unclear. To address this issue, we here investigate whether absence of GFAP affects the phenotypic expression of motor neuron disease (MND) using an H46R mutant Cu/Zn superoxide dismutase-expressing mouse model of ALS (SOD1(H46R)). GFAP deficient SOD1(H46R) mice showed a significant shorter lifespan than SOD1(H46R) littermates. Further, at the end stage of disease, loss of GFAP resulted in increased levels of Vim and Aif1 mRNAs, encoding vimentin and allograft inflammatory factor 1 (AIF1), respectively, in the spinal cord, although no discernible differences in the levels and distribution of these proteins between SOD1(H46R) and GFAP-deficient SOD1(H46R) mice were observed. These results suggest that loss of GFAP in SOD1(H46R) mice marginally accelerates the disease progression by moderately enhancing glial cell activation. Our findings in a mouse model of ALS may have implication that GFAP is not necessary for the initiation of disease, but it rather plays some modulatory roles in the progression of ALS/MND. SN - 1872-8111 UR - https://www.unboundmedicine.com/medline/citation/21453731/Loss_of_glial_fibrillary_acidic_protein_marginally_accelerates_disease_progression_in_a_SOD1_H46R__transgenic_mouse_model_of_ALS_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0168-0102(11)00094-0 DB - PRIME DP - Unbound Medicine ER -